Extramammary Paget disease (EMPD) is a rare skin condition that generally affects areas with abundant sweat glands, such as anogenital (areas surrounding the anus and genitals) or axillary (the armpit area) skin. It is often classified as an intraepithelial adenocarcinoma or adenocarcinoma in situ (AIS), which is a precancerous condition that shows abnormal cells within the top layer of the skin and may progress to cancer if left untreated.
EMPD can be classified into two categories, based on where the disease originated from. The first category is primary EMPD, which originates from the skin. The second category is secondary EMPD, which is associated with the metastasis of a primary adenocarcinoma originating somewhere else in the body.
EMPD is most commonly diagnosed in people over 50 and is more prevalent in females. However, this disease can be developed at any age in any gender.
Treatment
When cancers are detected, they are staged and graded based on size, metastasis, and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you. However, because EMPD is generally classified as a premalignant condition, the disease is generally not staged or graded. Instead of staging and grading, your doctor will recommend a treatment plan based on the following factors:
- Disease location.
- Whether or not the disease has progressed to cancer.
- Your age.
- General health.
- Your treatment preferences.
Treatment is dependent on several factors, including location, stage of disease and overall health.
Treatment options for EMPD may include:
- Surgery to remove as much of the tumour as possible, potentially including Mohs Micrographic surgery.
- Radiation therapy.
- Chemotherapy.
- Clinical trials.
- Palliative care.
Risk factors
Because of how rare EMPD is, there has been limited research into the risk factors of this disease.
Symptoms
The symptoms of EMPD are often confused with other skin conditions, and as such is often misdiagnosed. The most common occurrence of EMPD is the vulva in women and on the scrotum or around the anus in men. Common symptoms of this disease are:
- Itchiness of the skin.
- Burning, pain, tenderness or irritation.
- Skin discolouration around the area.
- Crusting and/or scaling lesions of the skin.
- Weeping lesions of the skin.
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Diagnosis
If your doctor suspects you have an EMPD, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment:
- Physical examination.
- Imaging tests, most commonly an ultrasound.
- Blood tests.
- Endoscopy, potentially including:
- Colonoscopy (for perianal EMPD).
- Cystoscopy (for EMPD of the urethra).
- Biopsy.